ISSN:
1432-1920
Keywords:
Magnetic resonance imaging
;
Pelizaeus
;
Merzbacher disease
;
Cerebral sclerosis diffuse
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary Pelizaeus-Merzbacher's disease is a progressive encephalopathy with demyelination of the cerebral white matter. The diagnosis can not be made on clinical or biological grounds: pathological investigation is necessary to confirm tigroid demyelination. CT scanning failed to visualize this type of anomaly but detection is now possible with the advent of magnetic resonance imaging (MRI). The authors studied the case of a boy who, at the age of 8 presented with symptoms characteristic of the disease: rotatory nystagmus, progressive encephalopathy, and inherited X-linked recessive traits. Magnetic resonance imaging revealed a high signal in the supra-tentorial white matter and the usual contrast was inverted. The authors believe that MRI can make an important contribution to the diagnosis of the disease.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00348925
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